Hemoglobin-Oxygen Equilibrium in Cystic Fibrosis

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Abstract
A study of 35 patients with cystic fibrosis demonstrated that increasing severity of pulmonary involvement was associated with a mild but definite increase in erythrocyte 2,3-diphosphoglycerate (2,3-DPG) and a decrease in Hb affinity for O2. The predominant regulators of 2,3-DPG were blood pH, cardiac output and systemic O2 transport. No significant relationship was observed between erythrocyte 2,3-DPG content and arterial O2 tension. Hypophosphatemia may have prevented a greater increase in erythrocyte 2,3-DPG content. The inadequate increase in 2,3-DPG and consequent insufficient change in Hb O2 affinity, coupled with an insufficient compensatory erythrocytic response, may adversely affect tissue oxygenation in patients with severe cystic fibrosis.